Characteristics of depression in Parkinson's disease: evaluating with Zung's Self-Rating Depression Scale.

The purpose of the study was to elucidate characteristics of depression in Parkinson's disease (PD). Fifty-eight PD patients were evaluated with Zung's Self-Rating Depression Scale (SDS) and the Unified Parkinson's Disease Rating Scale (UPDRS). Scores for "suicidal ideation" on the SDS correlated with posture and gait disturbances on the UPDRS. Twenty-six patients with spinocerebellar degeneration (SCD) were also evaluated with the SDS. SDS scores for "indecisiveness" and "constipation" were significantly higher in PD patients than SCD patients. Our results suggest that depression is common in disabled persons but PD patients might have a characteristic clinical presentation.

Elevation of cerebrospinal fluid protein in patients with diabetes mellitus is associated with duration of diabetes.

We investigated the relationships between total cerebrospinal fluid (CSF) protein in diabetic patients and the clinical characteristics of diabetes mellitus. The subjects comprised 16 diabetic patients (median age = 60.5 years, range = 47-71) who were studied retrospectively. Patients with diseases known to be associated with increases in total CSF protein were excluded as far as possible. The median total CSF protein and albumin quotient in the diabetic group were 52.5 mg/dl (range = 41-84) and 6.13 x 10(-3) (range = 4.0 x 10(-3) to 13.1 x 10(-3)), respectively. These results were significantly higher than those in 28 age-matched nondiabetic patients (median = 59.0 years, range = 50-71) (p < 0.01). Duration of diabetes was associated with total CSF protein (r = 0.642, p < 0.01). If the CSF shows increased total protein in a diabetic patient who has not suffered from long-term diabetes (> or =5 years), causes other than diabetes should be considered to explain increases in total CSF protein. We need to confirm the present results by studying a larger population of diabetic patients.

[Case of LGMD2A (calpainopathy) clinically presenting as Miyoshi distal myopathy].

We reported a 23-year-old woman with distal myopathy and highly elevated serum creatine kinase (CK) caused by calpainopathy. Although muscle weakness was not evident, a muscle CT scan revealed replacement by adipose tissue in the medial head of the gastrocnemius. The gluteus maximus and biceps femoris were also affected to a lesser degree, but the lateral head of the gastrocnemius was preserved. A histological study of a biopsied specimen of the biceps brachii revealed obvious variation in fiber size and a few necrotic or regenerating fibers. Rimmed vacuoles or lobulated fibers were absent in vacuoles. Although the clinical features suggested Miyoshi's distal myopathy, gene analysis of calpain 3 revealed a c.802-9G > A mutation in intron 5 and a c.1319G > A (p.Arg440Gln) in exon 10. Mini-multiplex Western Blotting (MMW) of the patient's muscle showed no band in calpain 3 (p94) and calpain 3 30 kDa fragments and immunoblotting did not reveal any dysferlin abnormalities. Calpainopathy should be also considered in patients with clinical manifestations of Miyoshi distal myopathy.

[Computed tomography (CT) angiography with multidetector row helical CT for detecting spinal dural arteriovenous fistula].

A 59-year-old man had have dysuria and left thigh pain for 2 months. He also experienced transient weakness of both legs twice, which recovered within a few hours. One month ago, he had bowel disturbance. Lumbar MRI showed a swelling of the conus medullaris. Enlarged and tortuous vessels behind the spinal cord on lumbar MRA and CT myelography was demonstrated. Though angiogram through intercostal and lumbar arteries revealed no abnormal findings, CT angiography with multidetector row helical CT (MDCT) showed abnormal vessel in intradural space at sacral level. Angiogram through left internal iliac artery revealed abnormal vessel fed by branches of the lateral sacral artery at the early arterial phase. Thus, diagnosis of dural arteriovenous fistula (DAVF) at the left S1 level was confirmed. It was indicated that CT angiography with MDCT was useful in detecting spinal DAVF especially at sacral level.

Hepatocellular carcinoma metastasizing to the skull base involving multiple cranial nerves.

We describe a rare case of HCV-related recurrent multiple hepatocellular carcinoma (HCC) metastasizing to the skull base involving multiple cranial nerves in a 50-year-old woman. The patient presented with symptoms of ptosis, fixation of the right eyeball, and left abducens palsy, indicating disturbances of the right oculomotor and trochlear nerves and bilateral abducens nerves. Brain contrast-enhanced computed tomography (CT) revealed an ill-defined mass with abnormal enhancement around the sella turcica. Brain magnetic resonance imaging (MRI) disclosed that the mass involved the clivus, cavernous sinus, and petrous apex. On contrast-enhanced MRI with gadolinium-chelated contrast medium, the mass showed inhomogeneous intermediate enhancement. The diagnosis of metastatic HCC to the skull base was made on the basis of neurological findings and imaging studies including CT and MRI, without histological examinations. Further studies may provide insights into various methods for diagnosing HCC metastasizing to the craniospinal area.

[Adult onset Langerhans cell histiocytosis with progressive cerebellar ataxia and spastic paraparesis].

A 44-year-old woman with progressive cerebellar ataxia and spastic paraparesis was referred to our hospital. Brain MRI showed bilateral high signals in superior, middle, and inferior cerebellar peduncles on the T2 weighted images. After 3 years, her symptoms progressively worsened in spite of various therapies including whole brain irradiation and high dose oral prednisone. No evidence of diabetes insipidus was noted. In MRI, brainstem lesions expanded to both hemispheres of the cerebellum without enhancement by contrast medium. We confirmed diagnosis of LCH by skin biopsy of intractable truncal rash which emerged after neurological symptoms.

[Intermittent intravenous immunoglobulin infusion prevented relapses in patients with remission-exacerbation type chronic inflammatory demyelinating polyradiculoneuropathy].

The intravenous immunoglobulin infusion therapy (IVIg) has recently acquired an important role in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Some patients, however, require repetitive infusions to maintain the improvement. We planned a one-day therapy with 0.4 g/kg of IVIg in every 7 or 10 days for two CIDP patients who had required a 5-day course of IVIg in every month because of frequent exacerbations. Serum levels of IgG in both patients were kept as high as 2,000 mg/dl resulting in maintaining the improvement without any side effects.

Hepatic encephalopathy in primary human immunodeficiency virus type 1 (HIV-1) infection.

Primary infection of human immunodeficiency virus type 1 (HIV-1) is occasionally associated with common cold-like symptoms, and rarely with a self-limited illness resembling infectious mononucleosis. We report a 32-year-old man who presented with infectious mononucleosis-like blood picture on admission. Five days after admission he developed hepatic encephalopathy, which was ameliorated by administration of bolus corticosteroid. Based on the results of serologic studies, we diagnosed that he had primary HIV-1 infection. To our knowledge, this is the first published report of hepatic encephalopathy as a clinical manifestation of primary HIV-1 infection.

[A 68-year-old man of dural arteriovenous fistula at the cranio-cervical junction with dysesthesia ascending from his both toes].

We reported a 68-year-old man of dural arteriovenous fistula at the cranio-cervical junction with dysesthesia ascending from his both toes. He recognized dysesthesia at his both toes 10 months previously. Thereafter dysesthesia ascended to his girdle which was stronger as far as his girdle and gait disturbance developed. Somatosensory evoked potential (SEP) revealed delayed central conduction time. Cervical MRI showed a swelling of the spinal cord and intramedullary hyperintense lesion from the C2 to C7 level on the T2-weighted image. Moreover flow void behind the mudulla oblongata on the T2-weighted MRI was outstanding. Angiogram through right ascending pharyngeal artery revealed enlarged and tortuous anterior and posterior spinal veins at the early arterial phase. We diagnosed as dural arteriovenous fistula (AVF) and conducted intraarterial embolization. After treatment, the swelling and hyperintense lesion of the cervical spinal cord improved on MRI, and flow void behind medulla oblongata was extinguished. Gait disturbance also improved. We think that the valves of veins in the spinal cord are responsible for the tendency of higher venous pressure in outer circumference, which results in the symptom dominating in the lower extremities. We recommend that dural AVF at the cranio-cervical junction should be considered as a differential diagnosis in case with the similar clinical course to our case.

[Effective thrombolytic therapy for calcified cerebral embolism originating from a calcified plaque in the internal carotid artery].

A 72-year-old man was transported to our emergency department after rear-ending another vehicle. He presented with acute left hemispatial neglect, left hemianopsia, and mild left hemiparesis. Computed tomography (CT) on admission showed a calcified embolus in the right middle cerebral artery. After intravenous thrombolytic therapy, the patient showed drastic improvement of neurological deficits. Follow-up CT showed disappearance of embolus, but distal migration of emboli to the downstream of the right middle cerebral artery was seen, sparing the massive territory of the right middle cerebral artery. Carotid duplex sonography and 3-dimensional CT angiography showed a calcified plaque with ulceration at the origin of the right internal carotid artery, representing the presumptive origin of the emboli. We report a rare case of effective intravenous thrombolysis for calcified cerebral embolism from the carotid artery. Further consideration of the mechanism, efficacy, and indication of intravenous thrombolysis for calcified cerebral emboli is needed.